Rosenbohm Angela, Kassubek Jan, Weydt Patrick, Marroquin Nicolai, Volk Alexander E, Kubisch Christian, Huppertz Hans-Jürgen, Weber Markus, Andersen Peter M, Weishaupt Jochen H, Ludolph Albert C
Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081, Ulm, Germany.
J Neurol. 2014 Feb;261(2):283-90. doi: 10.1007/s00415-013-7185-7. Epub 2013 Nov 20.
A recent staging effort for amyotrophic lateral sclerosis (ALS) has demonstrated that the TDP-43 neuropathology may initiate focally in the motor cortex in the majority of patients. We searched our data bank for patients with lesions of the motor cortex which preceded disease onset. We performed a search of our patient- and MRI-data bank and screened 1,835 patients with amyotrophic lateral sclerosis for frontal lobe/motor cortex lesions. We found 18 patients with definite ALS who had documented and defined lesions of the motor cortex, which preceded the initial ALS symptoms by 8-42 years. In the vast majority (15/18) of the patients, the onset of ALS was closely related to the focal lesion since it started in a body region reflecting the damaged cortical area. The findings suggest that initial lesions to the motor cortex may be a contributing initiating factor in some patients with ALS or determine the site of onset in individuals pre-disposed to ALS.
最近一项针对肌萎缩侧索硬化症（ALS）的分期研究表明，在大多数患者中，TDP - 43神经病理学可能在运动皮层局部起始。我们在数据库中搜索了疾病发作前存在运动皮层病变的患者。我们对患者和MRI数据库进行了检索，筛查了1835例肌萎缩侧索硬化症患者的额叶/运动皮层病变情况。我们发现18例确诊为ALS的患者有记录且明确的运动皮层病变，这些病变在最初的ALS症状出现前8 - 42年就已存在。在绝大多数（15/18）患者中，ALS的发病与局灶性病变密切相关，因为它始于反映受损皮层区域的身体部位。这些发现表明，运动皮层的初始病变可能是一些ALS患者的促成起始因素，或者决定了易患ALS个体的发病部位。
